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大剂量静脉注射免疫球蛋白治疗自身免疫性溶血性贫血。

High-dose intravenous immunoglobulin in the treatment of autoimmune haemolytic anaemia.

作者信息

Majer R V, Hyde R D

机构信息

Haematology Department, Southampton General Hospital, Hants, UK.

出版信息

Clin Lab Haematol. 1988;10(4):391-5. doi: 10.1111/j.1365-2257.1988.tb01186.x.

Abstract

Conventional doses of intravenous immunoglobulin (i.v. Ig) (0.4 g/kg/day for 5 days) commonly produce a remission in immune thrombocytopenia (ITP) but have only rarely been successful in autoimmune haemolytic anaemia (AIHA). There are a few reports of higher doses of i.v. Ig being more effective in AIHA. We have treated two patients with AIHA with high-dose i.v. Ig (0.5 g/kg/day for 5 days). In one patient with an associated ITP a prompt rise in platelet count but no change in Hb concentration occurred. The second patient with AIHA associated with chronic lymphatic leukaemia showed a prompt response, with a rise in Hb concentration and fall in plasma bilirubin. The poor response to i.v. Ig seen in AIHA may be related to the expansion of the reticulo-endothelial system seen in AIHA but not ITP. Clearance of antibody-coated red cells and platelets may occur at different rates and/or sites in the reticulo-endothelial system and this may account for the differential response seen in case 1. Higher doses of i.v. Ig, in the range 0.5-1 g/kg/day for 5 days, are required in AIHA, particularly if significant splenomegaly is present, and may be effective in refractory cases.

摘要

常规剂量的静脉注射免疫球蛋白(静脉注射Ig)(0.4 g/kg/天,共5天)通常可使免疫性血小板减少症(ITP)缓解,但在自身免疫性溶血性贫血(AIHA)中很少成功。有少数报告称较高剂量的静脉注射Ig对AIHA更有效。我们用高剂量静脉注射Ig(0.5 g/kg/天,共5天)治疗了两名AIHA患者。一名合并ITP的患者血小板计数迅速上升,但血红蛋白浓度无变化。第二名合并慢性淋巴细胞白血病的AIHA患者显示出迅速反应,血红蛋白浓度上升,血浆胆红素下降。AIHA中静脉注射Ig反应不佳可能与AIHA中所见的网状内皮系统扩张有关,而ITP中未见此现象。抗体包被的红细胞和血小板在网状内皮系统中的清除可能以不同的速率和/或部位发生,这可能解释了病例1中所见的不同反应。AIHA需要更高剂量的静脉注射Ig,范围为0.5 - 1 g/kg/天,共5天,特别是如果存在明显脾肿大时,且可能对难治性病例有效。

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